The recently reevaluated high prevalence of healthy carriers (1/2,000 in UK) of variant Creutzfeldt-Jakob Disease (v-CJD), whose blood might be infectious, suggests that the evolution of this prion disease might not be under full control as expected. After experimental transfusion of macaques and conventional mice with blood derived from v-CJD exposed (human and animal) individuals, we confirmed in these both models the transmissibility of v-CJD, but we also observed unexpected neurological syndromes transmissible by transfusion: despite their prion etiology confirmed through transmission experiments, these original cases would escape classical prion diagnosis, notably in the absence of detectable abnormal PrP with current techniques. It is noteworthy that macaques developed an original, yet undescribed myelopathic syndrome associating demyelination and pseudo-necrotic lesions of spinal cord, brainstem and optical tract without affecting encephalon, which is rather evocative of spinal cord disease than prion disease in human medicine. These observations strongly suggest that the spectrum of human prion diseases may extend the current field restricted to the phenotypes associated to protease-resistant PrP, and may notably include spinal cord diseases.

最近重新评估了变种的克雅氏病（v-CJD）的健康携带者的高流行率（英国为1 / 2,000），其血液可能具有传染性，这表明这种病毒病的发生可能未得到预期的完全控制。在对猕猴和常规小鼠进行实验性输血后，从暴露于v-CJD的（人类和动物）个体中抽取血液，我们在这两个模型中均确认了v-CJD的可传播性，但我们还观察到了可通过输血传播的意想不到的神经系统综合征：尽管其病毒病原学通过传播实验证实，这些原始病例将摆脱传统的病毒诊断，特别是在当前技术无法检测到异常PrP的情况下。值得注意的是猕猴开发了原始的 尚未描述的脊髓病综合征，与脊髓，脑干和视神经道的脱髓鞘和假性坏死相关，而不会影响脑，这在人类医学中比起e病毒病更能唤起人们的脊髓疾病。这些观察结果强烈表明，人类病毒病的范围可能扩大了限于与蛋白酶抗性PrP相关的表型的电流领域，并且可能特别包括脊髓病。